Antinuclear Antibodies in Systemic Sclerosis: an Update

Clin Rev Allergy Immunol. 2020 Feb;58(1):40-51. doi: 10.1007/s12016-018-8718-8.

Abstract

Systemic sclerosis is an autoimmune disease characterized by fibrosis of skin and internal organs, vasculopathy, and dysregulation of immune system. A diagnostically important feature of immunological abnormalities in systemic sclerosis is the presence of circulating antinuclear antibodies, which may be detected in 90-95% of patients with either of the four main laboratory methods: immunofluorescence, enzyme-linked immunosorbent assay, immunodiffusion, and immunoblotting. There are several antinuclear antibodies specific for systemic sclerosis. These include antibodies against topoisomerase (anti-TOPO I), kinetochore proteins (ACA), RNA polymerase enzyme (anti-RNAP III), ribonuclear proteins (anti-U11/U12 RNP, anti-U1 RNP, anti-U3 RNP) and nucleolar antigens (anti-Th/To, anti-NOR 90, anti-Ku, antiRuvBL1/2, and anti-PM/Scl). Autoantibodies specific for systemic sclerosis have been linked to distinct clinical features. Therefore, detecting a particular antibody type is important in predicting a possible organ involvement and prognosis and may have an impact on monitoring and treatment.

Keywords: Antinuclear antibodies; Connective tissue diseases; Diagnosis; Immunology; Malignancy; Prognosis; Scleroderma; Systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Antibodies, Antinuclear / immunology*
  • Autoantibodies / immunology
  • Autoantigens / immunology
  • Autoimmunity*
  • Biomarkers
  • Diagnosis, Differential
  • Disease Susceptibility*
  • Humans
  • Molecular Diagnostic Techniques / methods
  • Molecular Diagnostic Techniques / standards
  • Prevalence
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / epidemiology
  • Scleroderma, Systemic / etiology*
  • Scleroderma, Systemic / metabolism

Substances

  • Antibodies, Antinuclear
  • Autoantibodies
  • Autoantigens
  • Biomarkers